Abstract:

Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis or meningofacial
angiomatosis, is characterised in its classical form by a congenital, usually unilateral, ‘portwine
stain’ (capillary naevus) on the face, convulsions, typical intracranial calcification and
some degree of mental retardation and hemiparesis. The clinical correlation of intractable
seizures with the presence of bilateral intracranial disease has management and prognostic
implications, thus making the presence of bilateral disease an important factor to all those
involved in the management of the child with Sturge-Weber syndrome.